Selection of Surgical Approach for Cerebellar Hemangioblastomas Based on Venous Drainage Patterns
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Selection of Surgical Approach for Cerebellar Hemangioblastomas Based on Venous Drainage Patterns Takashi Watanabe ( [email protected] ) University of Miyazaki Faculty of medicine https://orcid.org/0000-0002-3724-1962 Yuuki Suematsu Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Kiyotaka Saitou Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Go Takeishi Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Shinji Yamashita Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Hajime Ohta Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Kiyotaka Yokogami Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Hideo Takeshima Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan Research Article Keywords: cerebellum, cerebral veins, hemangioblastoma, surgical approach Posted Date: February 15th, 2021 DOI: https://doi.org/10.21203/rs.3.rs-229544/v1 License: This work is licensed under a Creative Commons Attribution 4.0 International License. Read Full License Version of Record: A version of this preprint was published at Neurosurgical Review on April 20th, 2021. See the published version at https://doi.org/10.1007/s10143-021-01544-y. Page 1/24 Abstract Cerebellar hemangioblastomas remain surgically challenging because of the narrow, deep surgical corridors and tumor hypervascularity. Various surgical approaches are used according to the location, but optimal approaches have not been established. We propose a system of surgical approaches based on the venous drainage systems to facilitate surgical planning and achieve acceptable neurological outcomes. Cerebellar hemangioblastomas were divided into vefour types based on the main drainage systems: suboccipital hemangioblastomas draining to the transverse sinus (TS) or torcular, tentorial hemangioblastomas draining to the tentorial and straight sinus, petrosal hemangioblastomas draining to the superior petrosal sinus (SPS),, and quadrigeminal hemangioblastomas draining to the galenic system, and tonsillar hemangioblastomas draining to the TS or torcular in conjunction with jugular bulb or SPS. Microsurgical approaches and patient outcome were retrospectively reviewed according to this classication. This study included 17 patients who underwent 21 operations for resection of 19 cerebellar hemangioblastomas, classied into 911 suboccipital, 4 tentorial, 2 petrosal, and 2 quadrigeminal, and 2 tonsillar. Standard suboccipital craniotomies were utilized for suboccipital hemangioblastomas, the occipital transtentorial approach (OTA) and supracerebellar infratentorial approach for tentorial hemangioblastomas, the retrosigmoid approach for petrosal hemangioblastomas, and OTA for quadrigeminal hemangioblastomas, and midline suboccipital approach for tonsillar hemangioblastomashemangioblastomass. Gross total resection was achieved in all patients except one. Two patients with large hemangioblastomas (tonsillarsuboccipital and quadrigeminal) required second- stage operation which nally achieved gross total removal. No single approach had a signicantly higher incidence of postoperative neurological decits. Selection of the optimum surgical approach for cerebellar hemangioblastomas was successful based on the main drainage systems. Understanding of tumor growth and extension with respect to the venous drainage system is critical to select the appropriate surgical approach. Introduction Central nervous system (CNS) hemangioblastoma is a relatively rare intracranial neoplasm accounting for 1.5-2.5% of CNS tumors, and can occur as a sporadic entity as well as in patients with von Hippel- Lindau (VHL) disease, an autosomal dominant inherited disorder.[10,14] CNS hemangioblastomas are benign, highly vascularized tumors classied as grade I according to the World Health Organization classication. Despite the benign histological features of the tumor, mass effect resulting from tumor progression, peritumoral cyst formation, and expanding peritumoral edema may cause morbidity and mortality.[34] Surgery is curative and the treatment of choice in most patients with clinical symptoms. The goal of surgery is complete removal of the lesion to prevent perioperative hemorrhage and to preserve surrounding neurovascular functions. CNS hemangioblastomas occur most frequently in the cerebellum followed by other specic CNS locations such as the retina, spinal cord, and brainstem.[6,13,34] Various surgical approaches for cerebellar hemangioblastoma are adopted according to tumor location.[1,7-11,15,20,21,33] Cerebellar Page 2/24 hemangioblastomas present unique operative challenges, despite current advances in microneurosurgical techniques, such as the narrow and deep surgical corridor in the posterior fossa, inability to debulk the tumor mass due to hypervascularity, diculty in early access to the feeding artery behind the tumor, and interference by large tortuous draining veins during the resection.[8-10,19,21] Consequently, selection of the optimal surgical approach and operative planning is quite important to access the pial surface and achieve panoramic exposure of the circumferential entire margin of the lesion for early management of the cortical feeding artery and identication of the main draining system. However, the optimal surgical approach to cerebellar hemangioblastomas has not been systematically established and comprehensively specied because of the rarity of these lesions. The current study tried to systematize our surgical approach and evaluated our surgical results for 19 cerebellar hemangioblastomas according to a classication of the cerebellar surface into ve different regions based on the venous drainage system. We found that these subtypes offer intuitive appreciation of their anatomy and surgical management and are useful in describing the surgical results. Materials & Methods Patient Population Nineteen consecutive patients underwent a total of 24 operations for resection of 22 cerebellar hemangioblastomas at the University of Miyazaki between January 2007 and March 2020. Three recurrent hemangioblastomas in 2 patients were excluded because the drainage veins could not be determined due to the previous surgery, so nally 17 patients who underwent a total of 21 operations for 19 cerebellar hemangioblastomas were evaluated. Medullary and spinal hemangioblastomas were not included in this study. The diagnosis of VHL disease was made according to the clinical criteria. Clinical data pertaining to patient demographics, neurological status, operative record, and clinical outcome were obtained by retrospective chart review. All available baseline medical data were assessed, including medical records, neuroimaging studies, intraoperative photographs, and videos. All patients underwent 3.0 T magnetic resonance (MR) imaging (contrast-enhanced T1-weighted and uid-attenuated inversion recovery [FLAIR] images), and three-dimensional computed tomography angiography to evaluate the tumor location, maximum tumor diameter, course of the feeding artery, and draining veins. Maximum tumor and/or cyst diameter were measured and classied into small (<25 mm) and large (≥25 mm) tumors. Intraoperative ndings were systematically reviewed on the operative records and videos. Extent of resection was determined by retrospective review of the postoperative MR images obtained within 3 days after surgery. Histological diagnosis was made according to the criteria of the World Health Organization classication.[4,14] Detailed neurological assessments were performed by neurosurgical residents and the senior neurosurgeon, preoperatively and postoperatively. Neurological outcome was assessed with the modied Rankin Scale (mRS). Good and pooroutcomes were denedas nal mRS ≤2 and mRS >2. Neurological improvement and deterioration were dened as decrease and Page 3/24 increase in mRS score, respectively. Sixteen hemangioblastomas with 14 patients were followed up for at least 5 months with serial clinical and MR imaging examinations at 6- or 12-month intervals. Classication of Cerebellar Hemangioblastomas Cerebellar hemangioblastomas were organized into the following ve groups according to their drainage venous system (Fig. 1). Suboccipital Hemangioblastoma Suboccipital hemangioblastoma is located on the suboccipital and tonsillar surface and drains toward the inferior vermian veins, inferior hemispheric veins, or both, which empty into the transverse sinus or torcular. Tentorial Hemangioblastoma Tentorial hemangioblastoma faces the tentorial surface and is located in the extreme narrow space along the supracerebellar-infratentorial plane. Venous drainage is through the superior hemispheric veins, which empty into the straight sinus or tentorial sinus. Petrosal Hemangioblastoma Petrosal hemangioblastoma faces the corner of the petrosal surface and tentorium, and drained by the petrosal veins, which empty into the superior petrosal sinus (SPS). Quadrigeminal Hemangioblastoma Quadrigeminal hemangioblastoma is located on the anteromedial cerebellar surface facing the quadrigeminal cistern, and drained by the precentral cerebellar vein or superior vermian vein, which belong to the galenic system that drains into the vein of Galen. Tonsillar Hemangioblastoma Tonsillar hemangioblastoma is located on the tonsillar surface and drains toward the supercial venous